A therapy with miglustat, 2-hydroxypropyl-ß-cyclodextrin and allopregnanolone restores splenic cholesterol homeostasis in Niemann-pick disease type C1

Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann–Pick-Disease Type C1

(2018) Lynn Ebner et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Hydroxypropyl-Beta-Cyclodextrin Reduces Inflammatory Signaling from Monocytes: Possible Implications for Suppression of HIV Chronic Immune Activation

(2018) Flávio Lemos Matassoli et al.   mSphere

FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann-Pick type C mutant fibroblasts

(2017) Jason Newton et al.   FASEB JOURNAL

Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1–2 trial

(2017) Daniel S Ory et al.   LANCET

Morphology of Niemann-Pick type C metabolic storage disorder

(2016) R. M. Ireland   BLOOD

Telocytes: a potential defender in the spleen of Npc1 mutant mice

(2016) Bichao Zhang et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

CD68/macrosialin: not just a histochemical marker

(2016) Dimitry A Chistiakov et al.   LABORATORY INVESTIGATION

Immune dysfunction in Niemann-Pick disease type C

(2015) Nick Platt et al.   JOURNAL OF NEUROCHEMISTRY

Plasticity-related Gene 5 Promotes Spine Formation in Murine Hippocampal Neurons

(2014) Pierluca Coiro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

An update on the biology of sphingosine 1-phosphate receptors

(2014) Victoria A. Blaho et al.   JOURNAL OF LIPID RESEARCH

Reduced cerebellar neurodegeneration after combined therapy with cyclodextrin/allopregnanolone and miglustat in NPC1: A mouse model of Niemann-Pick type C1 disease

(2014) Fabian Maass et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Olfactory Deficits in Niemann-Pick Type C1 (NPC1) Disease

(2014) Marina Hovakimyan et al.   PLoS One

Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1

(2013) A. O. Speak et al.   BLOOD

The Spleen in Local and Systemic Regulation of Immunity

(2013) Vincenzo Bronte et al.   IMMUNITY

Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling

(2013) Martin Fan et al.   JOURNAL OF LIPID RESEARCH

Pre-Symptomatic Activation of Antioxidant Responses and Alterations in Glucose and Pyruvate Metabolism in Niemann-Pick Type C1-Deficient Murine Brain

(2013) Barry E. Kennedy et al.   PLoS One

Sphingosine-1-Phosphate and Lymphocyte Egress from Lymphoid Organs

(2012) Jason G. Cyster et al.   Annual Review of Immunology

Autophagy in lysosomal storage disorders

(2012) Andrew P. Lieberman et al.   Autophagy

NPC1 defect results in abnormal platelet formation and function: studies in Niemann–Pick disease type C1 patients and zebrafish

(2012) Sophie Louwette et al.   HUMAN MOLECULAR GENETICS

Cyclodextrin mediates rapid changes in lipid balance inNpc1−/−mice without carrying cholesterol through the bloodstream

(2012) Anna M. Taylor et al.   JOURNAL OF LIPID RESEARCH

Modulators of Sphingolipid Metabolism Reduce Lung Inflammation

(2011) Maria Cristina Dechecchi et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Immune Regulation by Sphingosine 1-Phosphate and Its Receptors

(2011) Constantin Bode et al.   ARCHIVUM IMMUNOLOGIAE ET THERAPIAE EXPERIMENTALIS

Npc1 deficiency in the C57BL/6J genetic background enhances Niemann–Pick disease type C spleen pathology

(2011) Julio Parra et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Follicular dendritic cells help establish follicle identity and promote B cell retention in germinal centers

(2011) Xiaoming Wang et al.   JOURNAL OF EXPERIMENTAL MEDICINE

The sphingosine 1-phosphate receptor S1P2 maintains the homeostasis of germinal center B cells and promotes niche confinement

(2011) Jesse A Green et al.   NATURE IMMUNOLOGY

Innate or Adaptive Immunity? The Example of Natural Killer Cells

(2011) E. Vivier et al.   SCIENCE

Determination of reference genes for circadian studies in different tissues and mouse strains

(2010) Rok Kosir et al.   BMC MOLECULAR BIOLOGY

Assessment of splenic function

(2010) A. P. N. A. Porto et al.   EUROPEAN JOURNAL OF CLINICAL MICROBIOLOGY & INFECTIOUS DISEASES

The Beneficial Effects α-Cyclodextrin on Blood Lipids and Weight Loss in Healthy Humans

(2010) Kevin B. Comerford et al.   Obesity

Niemann-Pick disease type C

(2010) Marie T Vanier   Orphanet Journal of Rare Diseases

Lipids on Trial: The Search for the Offending Metabolite in Niemann-Pick type C Disease

(2010) Emyr Lloyd-Evans et al.   TRAFFIC

The clinical spectrum of fetal Niemann-Pick type C

(2009) Ronen Spiegel et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Inhibitory Role of Sphingosine 1-Phosphate Receptor 2 in Macrophage Recruitment during Inflammation

(2009) J. Michaud et al.   JOURNAL OF IMMUNOLOGY

Lack of Niemann–Pick type C1 induces age-related degeneration in the mouse retina

(2009) Thomas Claudepierre et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

(2009) Cristin D. Davidson et al.   PLoS One

Substrate reduction therapy

(2008) Frances M Platt et al.   ACTA PAEDIATRICA